ESPE Abstracts

Background: Cystic fibrosis is associated with abnormal lipid metabolism and this anormality is commonly characterized by low cholesterol and hypertriglyceridemia. The increasing in life expectancy of cystic fibrosis patients has enhancing the interest for prevent the risk factors for cardiovascular diseases like dyslipidemias.

Objective and hypotheses: Determine whether concentrations of cholesterol and triacylglycerol are related to nutritional status and fasting glucose in pediatric patients with cystic fibrosis.

Method: Fasting lipid profiles and fasting glucose were measured in 52 pediatric patients with cystic fibrosis (10.5±5.0 years – 23F/29M).

Results: Twenty patients (38.4%) had hypertriglyceridemia (143.4±37.8 mg/dl), and nine patients (17.3%) had elevated cholesterol (162±15 mg/dl). In most cases, hypertriglyceridemia was isolated; only three subjects had elevation of both cholesterol and hypertriglyceridemia. Twenty-eight patients (53.8%) had low HDL-cholesterol (34.8±6.5 mg/dl). Lipid concentrations were not related to BMI, gender or age or fasting glucose. These results have showed a group with higher triacylglycerol, lower cholesterol, and lower HDL-cholesterol concentrations than the general Brazilian pediatric population and also in comparison with some other studies in cystic fibrosis group.

Conclusion: Isolated hypertriglyceridemia appears to be common in cystic fibrosis, whereas cholesterol concentrations are generally low. Whether abnormal lipid metabolism is associated with a high risk of cardiovascular disease in general population, like elevation in cholesterol and triacylclycerol and low HDL-cholesterol, these findings arouse an importance to understand the abnormalities in lipid metabolism in this special group of children with cystic fibrosis in order to prevent the factors of risk of cardiovascular diseases and to improve the survival.